Introduction Endometrial stromal sarcoma (ESS) is an uncommon malignant mesenchymal tumor. Uterine localization is frequently seen but low grade ESS arising in extrauterine sites is so rare. Clinical presentation of this entity is no specific. So that, the diagnosis is based on histological analysis.

Methods We report two cases of low garde extrauterine endometrial stromal sarcoma treated at Salah Azaiez Institute.

Results The first case was a premenopausal female presented with abdominal pain. MRI examination showed an abominopelvic mass. She had right salpingooophorectomy. Microscopic analysis showed a low graden endometrial stromal sarcoma probably arising from endometriosis. Six months after, CT scan revealed pelvic recurrence depending on the ovary. She had hysterectomy with left salpingooophorectomy, omentectomy and appendicectomy. Histological report concluded on low graden ESS of ovary arising from endometriosis. The second case was a postmenopausal women with the history of hysterectomy with bilateral salpingoophorectomie for benign fibroma presented with pelvic pain. CT scan showed two abdominal mass. Peroperatory, there were three mass depending on the mesorectum, the mesentery and the epiplon. Dissection was difficult. Epiploic biospy was taken. After histological and immunohistochemical study, the diagnosis of low grade extrauterine endometrial stromal sarcoma was retained. Hormonal therapy with regular follow up were indicated.

Conclusion Extrauterine localisation is reported only is isolated cases in the littérature. In the absence of uterine primary tumor, the differential diagnosis considerations depend upon the site of involvement. Surgical resection or debulking are the mainstay of treatment.